title: Dysfunctions within limbic-motor networks in amyotrophic lateral sclerosis.
abstract: Previous studies have shown that affective symptoms are part of the clinical picture in amyotrophic lateral sclerosis (ALS), the most common motor neuron disorder in elderly people. Diffuse neurodegeneration of limbic regions (e.g., prefrontal cortex [PFC], amygdala) was demonstrated in ALS post-mortem, although the mechanisms of emotional dysregulation in ALS in vivo remain unclear. Using functional imaging, we assessed the brain responses to emotional faces in 11 cognitively unimpaired ALS patients and 12 healthy controls (HCs). We tested whether regional activities and connectivity patterns in the limbic system differed between ALS patients and HCs and whether the variability in clinical measures modulated the neuroimaging data. Relative to HCs, ALS patients displayed greater activation in a series of PFC areas and altered left amygdala-PFC connectivity. Anxiety modulated the right amygdala-PFC connectivity in HCs but not in ALS patients. Reduced right premotor cortex activity and altered left amygdala-supplementary motor area connectivity were associated with longer disease duration and greater disease severity, respectively. Our findings demonstrate dysfunctions of the limbic system in ALS patients at early stages of the disease, and extend our knowledge about the interplay between emotional brain areas and the regions traditionally implicated in motor control.
authors.affiliation: Consiglio Nazionale delle Ricerche, Istituto di Scienze Neurologiche, Unità di Ricerca Neuroimmagini, Catanzaro, Italia. luca.passamonti@cnr.it
authors.forename: Luca
authors.initials: L
authors.lastname: Passamonti
authors.forename: Francesco
authors.initials: F
authors.lastname: Fera
authors.forename: Alessandro
authors.initials: A
authors.lastname: Tessitore
authors.forename: Antonio
authors.initials: A
authors.lastname: Russo
authors.forename: Antonio
authors.initials: A
authors.lastname: Cerasa
authors.forename: Cecilia M
authors.initials: CM
authors.lastname: Gioia
authors.forename: Maria R
authors.initials: MR
authors.lastname: Monsurrò
authors.forename: Raffaella
authors.initials: R
authors.lastname: Migliaccio
authors.forename: Gioacchino
authors.initials: G
authors.lastname: Tedeschi
authors.forename: Aldo
authors.initials: A
authors.lastname: Quattrone
mesh_terms: D000328:Adult; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications / Q000473:pathology*; D001931:Brain Mapping; D003072:Cognition Disorders / Q000209:etiology; D005260:Female; D007839:Functional Laterality; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008032:Limbic System / Q000098:blood supply / Q000473:pathology*; D016014:Linear Models; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D019964:Mood Disorders / Q000209:etiology / Q000473:pathology; D009483:Neuropsychological Tests; D010100:Oxygen / Q000097:blood
chemical_list: D010100:Oxygen
keywords: Amygdala; Faces; Motor neuron disease; Prefrontal cortex; fMRI